PB2675: QUALITY OF LIFE IN INDIAN ADULTS WITH INHERITED BLEEDING DISORDERS
Authors: Samudyata Satish, Vivek Bhat, Dhwani Ravi, Chaitanya H Balakrishnan, Tiji Alphonse, Sanjukta S Rao, Seetharam Anandram, Cecil Ross
Journal: HemaSphere (2023)

Topic: 35. Quality of life and palliative care Background: ‘Inherited bleeding disorder’ (IBD) is an umbrella term referring to inherited deficiencies of coagulation factors. This includes hemophilia, the most common, as well as other, rarer deficiencies. These disorders typically require lifelong treatment, making quality of life (QOL) an important clinical consideration. Prior studies, predominantly based in Western countries, have demonstrated impaired QOL in patients with IBDs, stemming from both disease-related and treatment-related concerns. However, data on the QOL of Indian patients are scarce. Aims: This study aimed to assess the QOL, as well as the factors affecting QOL, of adult patients with IBDs at a tertiary care center in South India. Methods: We conducted this cross-sectional study at a tertiary care, referral hematologic center in Bangalore, South India. We included all adult patients with IBDs, from August 2022 to February 2023. After collecting their demographic details, we administered the Haem-A-QOL questionnaire in their language of choice. Haem-A-QOL is a validated questionnaire that has been used to assess QOL in hemophilia as well as other IBDs. It consists of 46 questions covering 10 different domains – physical health, feeling, opinion about oneself, sports & leisure, work & studies, dealing with IBDs, dealing with IBD treatment, future, planning family, partnership & sexuality. Each question was scored on a five-point Likert scale, with five indicating the most QOL impairment. Overall and domain-specific QOLs were calculated as a percentage of the maximum possible score, with greater values indicating worse QOL. We defined a score of >50 as poor, and >70 as very poor QOL. We performed relevant descriptive and inferential analyses on SPSS V22, taking a p-value of <0.05 as statistically significant for the latter. Results: We included 90 patients, of whom 89% (N=80) were male. The mean (SD) age was 31.6 (10.9) years. The most common diagnosis was hemophilia A, seen in 68.8% (N=62) followed by hemophilia B (12.2%, N=11), with other diagnoses including afibrinogenemia, von Willebrand disease, inherited multiple factor deficiency, and other isolated factor deficiencies. Among those with hemophilia A, 70.9% (N=44) had severe disease. The mean overall QOL score was 58.3 (11.7). Among domains, sports & leisure was the domain with the worst mean QOL, with a mean score of 69.7 (6.9), while partnership & sexuality had the greatest QOL, with a mean score of 46.7 (11.7) (Figure 1). 78.9% (N=71) had poor, and 20% (N=18) had very poor QOL. Males were more likely to have worse QOL (p=0.020), potentially explained by our finding that patients with hemophilia A, B, or C were more likely to have worse QOL compared to other diagnoses (p=0.007). There was no statistically significant difference in QOL based on age, household income, education level, or number of IBD-related inpatient admissions. Among those with hemophilia, there was no statistically significant difference in QOL based on type (A, B, or C), or severity. Summary/Conclusion: Our study provides a valuable, albeit preliminary, quantitative estimate of QOL in adult Indian patients with IBDs. Treating hematologists must be aware of impaired QOL in patients with IBDs, particularly hemophilia. Our findings may encourage physicians to further consider QOL in clinical decision making, and support them in providing more personalized care.Keywords: Bleeding disorder, Quality of life